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Query: UMLS:C0027960 (mole)
 21,279 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A retrospective study was undertaken in 47 children with congenital capillary naevus of the face in order to appreciate the predictive value of the topography of the facial angioma for the diagnosis of associated pial angioma. In patients with Sturge-Weber disease, facial angioma concerned the whole upper eyelid, extending to the forehead and/or cheek. Less often, it was localized to the root of the nose; in such cases it was a port-wine, homogeneous angioma, with clearcut outlines, quite different from the angiomatous dots frequently seen in this area in neonates. Except for these rare cases, angiomas concerned only half or less of the upper eyelid and no pial angioma was associated. Finally, congenital capillary naevi with this topography typical of Sturge-Weber disease were frequently associated with pial angioma.
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PMID:[Sturge-Weber disease: value of the topographic analysis of the facial angioma for the diagnosis of associated pial angioma (author's transl)]. 710 71

A 5-year-old female patient with a left facial strawberry nevus, ipsilateral optic atrophy and cerebellar signs was observed. Computed tomography and cerebral angiography confirmed left cerebellar hypoplasia and cerebrovascular abnormalities such as internal carotid arterial stenosis, basilar artery occlusion, and a saccular aneurysm of left carotid siphon, all of which are presumed to develop congenitally. This case may represent a new variety of neurocutaneous syndrome which is different from other syndromes associated with facial hemangioma, such as the Sturge-Weber syndrome and the Wyburn-Mason syndrome.
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PMID:Facial hemangioma with cerebrovascular anomalies and cerebellar hypoplasia. 713 14

Two neonates with the Kasabach-Merritt syndrome (cavernous haemangioma and spontaneous bleeding) are described. The first bled into a rapidly growing cervical haemangioma. A consumptive coagulopathy was found, and he required blood transfusions; no response to steroids was observed. Further treatment was conservative, and at the age of 2 years he is well and the haemangioma is shrinking. The second infant had a strawberry naevus on the thigh which started bleeding when he was 10 days old, a buttock mass and a mass in the foot, thought to be lipomas. No clear benefit was derived from steroids. Bandaging of the thigh prevented further bleeding, and he is well at the age of 10 months. Conservative management seems best in the condition, and steroids appear to be of value in approximately 40% of cases.
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PMID:Conservative management of the Kasabach-Merritt syndrome (cavernous haemangioma and thrombocytopenia). 719 3

The case of an 8-year-old boy presenting diffuse, prevalently right-sided nevus flammeus, venous angioma of the right cerebral hemisphere, right-sided facial hyperplasia, lef-handedness and recurring strokes is reported together with the clinical, angiographic and CT features.
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PMID:Cerebral venous angioma, cutaneous angioma, facial asymmetry and recurrent stroke. A case report. 720 40

A caruncular swelling with light brown pigmentation suggests a compound or junctional nevus. A proliferation of mucous cells is very often found inside this nevus (cases 1 and 2). A yellowish lobular tumor of the caruncle is found in hyperplasia of the sebaceous glands (case 3). Adenomata of the lacrimal glands and oncocytomata may be of a glazed reddish color (case 4), whereas a red caruncle suggests a hemangioma (case 5).
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PMID:[Hyperplasias of mucous, sebaceous and lacrimal glands and of blood vessels at the caruncula lacrimalis (author's transl)]. 723 Jun 72

Using only a few tests, it was possible to differentially diagnose a benign choroidal nevus with overlying medullation. Having revealed a flat retina with no apparent mass in the area, the ultrasound B-scan initially ruled out a choroidal malignant melanoma, choroidal hemangioma, metastatic tumor and a rhegmatogenous retinal detachment. Binocular indirect ophthalmoscopy and application of the Goldmann 3-mirror gonioscopy lens revealed that the lesion was not a retinal detachment. Due to the location of the lesion, disciform macular degeneration was also ruled out. Fluorescein angiography was the final test which aided in the definitive differential diagnosis, since it was characteristic of a choroidal nevus. Even though not all possible choroidal nevi need such an extensive work-up, occasionally specialized test can aid in the differential diagnosis of benign from potentially malignant lesions.
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PMID:Choroidal pigmentation - a differential diagnosis. 726 44

Two cases of Sturge-Weber-Krabbe's syndrome with unusual symptomatology are described. In the first case 13 years old boy the naevus flammeus was missing. A convulsive fit at the age of 2 1/2 years led to the correct diagnosis from the very characteristic radiological findings. At 13 years, intracortical calcifications were bulky and diffuse, and associated with optic nerve hypoplasia and anomalies of ocular motricity, their relation with the syndrome being difficult to establish. In the second patient (aged 17) the naevus flammeus was extensive, with numerous naevi on the whole body, and was associated with a retinal vascular malformation and glaucoma. A choroidal angioma was not observed. In these two patients the disease was isolated, without familial character, and there were no other neurological symptoms such as a pathological EEG. No evidence of mental retardation was noted.
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PMID:[Sturge-Weber-Krabbe syndrome with unusual symptomatology (author's transl)]. 728 82

A review of 373 patients with presumed choroidal nevi revealed 19 (5.1%) to have largely or totally amelanotic nevi. All patients but one with amelanotic nevi were white, and all were 48 years of age or older. Each lesion was located posterior to the equator, had a mean diameter of 3.2 mm, and was less than or equal to 1 mm in elevation. Intravenous fluorescein angiography most often disclosed early and late hyperfluorescence, corresponding to the amelanotic areas of the nevus. No lesions were observed to grow during a mean 14-month follow-up period. The differential diagnosis of amelanotic nevi of the choroid is discussed and includes amelanotic malignant melanoma, cavernous hemangioma of the choroid, metastatic cancer to the choroid, posterior scleritis, choroidal osteoma, and hypopigmented congenital hypertrophy of the retinal pigment epithelium.
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PMID:Amelanotic choroidal nevi. 733 17

Two large studies from the Armed Forces Institute of Pathology (AFIP) have listed the various lesions which may clinically resemble malignant melanomas of the posterior uvea (pseudomelanomas). The studies from the AFIP were based upon histologic examination of eyes which were enucleated. The present study reports on 400 consecutive patients who were referred but who proved, by clinical evaluation, rather than by enucleation, to have a pseudomelanoma. Although about 40 different conditions were found to simulate melanoma, the more commonly encountered ones included suspicious choroidal nevus (26.5%), disciform degeneration (12.5%), peripheral disciform degeneration (11%), congenital hypertrophy of the retinal pigment epithelium (9.5%), and choroidal hemangioma (8%). The authors believe that this series provides the clinician with a differential diagnosis for posterior uveal melanomas which accurately reflects the clinical problem confronting ophthalmologists today.
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PMID:The differential diagnosis of posterior uveal melanoma. 741 40

Congenital and acquired cerebrovascular diseases in the very young age group are reviewed and discussed. Whilst saccular aneurysms are rare arteriovenous malformations and cavernous hemangiomas represent the most frequent types of all congenital anomalies. The aneurysm of the great vein of Galen manifests in the newborn period and mostly is mistaken for congenital heart disease. If the infant survives this angioma causes hydrocephalus. A particular type of phakomatosis combines intracerebral pure venous malformation with homolateral port-wine nevus on the front, often causing epileptic seizures. Spontaneous intracerebral hemorrhage occurs in children with small cavernous hemangiomas. Acquired arterial lesions may develop during fetal life by embolism, causing porencephaly or unilateral brain atrophy. The "Moyamoya" syndrome represents a frequent multi-arterial lesion causing characteristic ischemic episodes. Etiology is still obscure. We do not even know if the disease is acquired or congenital.
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PMID:Cerebrovascular diseases in the first three years of life. 743 79


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